Premium
Optimizing outcomes for neonatal ARPKD
Author(s) -
Beaunoyer Mona,
Snehal Mohile,
Li Li,
Concepcion Waldo,
Salvatierra Oscar,
Sarwal Minnie
Publication year - 2007
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/j.1399-3046.2006.00644.x
Subject(s) - medicine , nephrectomy , surgery , liver transplantation , dialysis , transplantation , kidney
A retrospective analysis was conducted on 10 consecutive cases of neonatal ARPKD, 9 of whom received kidney transplants (KT). All were diagnosed antenatally (n = 6) or at birth. In the first month of life 70% required ventilatory support. Pre‐emptive bilateral nephrectomy and peritoneal dialysis (PD) catheter placement were performed in 9 at a mean age of 7.8 ± 11.9 months. The indications for nephrectomy were massive kidneys, resulting in suboptimal nutrition and respiratory compromise. All patients received assisted enteral nutrition, with significant increase in mean tolerated feeds following nephrectomy (p < 0.05), with increase in mean normalized weight and height (0.92 and 1.2 delta SDS respectively), by one year post‐transplantation. KT was performed at a mean age and weight of 2.5 ± 1.4 years and 13.3 ± 6.1 kg. The mean creatinine clearance at one year post‐KT was 91.3 ± 38.1 mls/min/1.73 m 2 , with a projected graft life expectancy of 18.4 years. Patient survival was 89% and death censored graft survival was 100%, at a mean follow‐up of 6.1 ± 4.5 years post‐transplant. Six patients demonstrated evidence of hepatic fibrosis, one of which required liver transplantation. In patients with massive kidneys from ARPKD, pre‐emptive bilateral nephrectomy, supportive PD and early aggressive nutrition, can minimize early infant mortality, so that subsequent KT can be performed with excellent patient and graft survival.